Sleep disorders with neurological disease

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Target exams

Red flags

• A previously well child with irresistible daytime sleep and sudden emotion-triggered loss of muscle tone has cataplexy, which is pathognomonic for narcolepsy type 1 and demands urgent sleep specialist referral
• A child with neuromuscular disease or severe cerebral palsy who develops morning headache, daytime sleepiness, or morning confusion may have symptomatic nocturnal hypoventilation progressing to respiratory failure and needs urgent oximetry and capnography
• A nocturnal event that is stereotyped, brief, and occurs several times a night, or in more than the first third of the night, is more likely nocturnal frontal lobe epilepsy than a parasomnia and needs video-electroencephalography
• Witnessed apnoeas, snoring, and restless sleep in a child with cerebral palsy or Down syndrome signal obstructive sleep apnoea that can cause cor pulmonale and neurocognitive decline if untreated
• Restless sleep, bedtime resistance, and a family history of restless legs with a low ferritin point to restless legs syndrome, which a sedative will worsen but iron will help
• Chronic insomnia in a child with autism that persists despite good sleep hygiene should prompt consideration of prolonged-release melatonin, but never as a first-line substitute for behavioural measures

Life stages

Care settings

Clinical exam formats

Board mappings

• Recognises that sleep disturbance is bidirectional with neurological disease and screens every neurodisability and epilepsy patient with a sleep history and a validated questionnaire
• Distinguishes the common sleep disorders by cluster: dyssomnias, sleep-disordered breathing, parasomnias, and sleep-related movement disorders
• Initiates sleep hygiene and behavioural therapy and refers for polysomnography when sleep-disordered breathing or narcolepsy is suspected
• Justifies the targeted investigation pathway: polysomnography for breathing and movement disorders, multiple sleep latency test and cerebrospinal fluid orexin for narcolepsy, ferritin for restless legs
• Manages sleep-disordered breathing of cerebral palsy and neuromuscular disease from adenotonsillectomy through continuous positive airway pressure to non-invasive ventilation
• Distinguishes nocturnal frontal lobe epilepsy from disorders of arousal using video-electroencephalography and treats the cause
• The bidirectional relationship between sleep and neurological disease and the four-cluster classification
• Narcolepsy type 1: orexin loss, cataplexy, multiple sleep latency test criteria, and orexin below 110 pg per mL
• The stepped management from sleep hygiene through melatonin, iron, airway and ventilatory support, and specialist pharmacotherapy
• Takes a structured sleep history, uses a sleep diary and validated questionnaire, and performs a focused examination targeting airway, tone, and development
• Builds a stepped management plan with the family and explains that treating sleep improves seizures, behaviour, and cognition
• Coordinates shared care between primary paediatrics, neurodisability, epilepsy, and the sleep service
• Level 1: Recognition that sleep disorders are common and consequential in children with neurological disease
• Level 2: Sleep hygiene, behavioural therapy, melatonin for insomnia of autism, and iron for restless legs
• Level 3: Polysomnography, multiple sleep latency testing, and ventilatory support for complex neurodisability
• The four-cluster classification and the bidirectional mechanism of sleep and the brain
• Diagnostic criteria for narcolepsy (mean sleep latency 8 minutes or less, 2 or more sleep-onset REM periods, orexin below 110 pg per mL) and restless legs (low ferritin)
• Evidence base for melatonin in autism and intravenous ferric carboxymaltose in refractory restless legs
• Assessment of the sleepy, snoring, or restless child including cataplexy and the sleep diary
• Stepped management explained to a family, emphasising treatable drivers and safety
• Recognition of red flags: cataplexy, nocturnal hypoventilation, and possible nocturnal frontal lobe epilepsy
• American Academy of Sleep Medicine scoring rules and the apnoea-hypopnoea and periodic limb movement indices
• Melatonin trial evidence for insomnia of autism spectrum disorder and iron for restless legs
• Sleep-disordered breathing in cerebral palsy and the role of polysomnography and airway or ventilatory support
• Recognition of sleep disorders as common, consequential, and treatable in children with neurological disease
• Stepped management built on sleep hygiene, behavioural therapy, and targeted pharmacotherapy and ventilation
• Family communication around the bidirectional link between sleep, seizures, cognition, and behaviour
• Canadian approach to case-finding sleep disorders in the neurodisability and epilepsy clinic
• Polysomnography, multiple sleep latency testing, and targeted iron and melatonin therapy
• Counselling on the favourable prognosis when sleep disorders are recognised and treated, and the role of telehealth for remote families